Abstract
BackgroundLate-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3 months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. Among LONIPCs, we occasionally treat patients with late-onset severe restrictive lung defect after HSCT; however, its clinical features have not been fully elucidated.MethodsA retrospective chart review of a single center on cases of late-onset severe restrictive lung defect after HSCT was performed. Among 453 patients who survived longer than 100 days after allogeneic HSCT with evaluable spirometry data, 12 patients (2.6%) developed late-onset severe restrictive lung defect (i.e., vital capacity percent of predicted less than 60%).ResultsMedian duration from transplantation to diagnosis of late-onset severe restrictive lung defect cases was 44.5 months. Major computed tomography (CT) finding was pleuroparenchymal thickening with volume loss, an evidence of fibrosis, predominantly in upper lobes (n = 7), which was consistent with pleuroparenchymal fibroelastosis. The remaining patients showed unclassifiable interstitial pneumonia pattern (n = 2) and airway-predominant pattern (n = 3). The diffusing capacity for carbon oxide tended to decrease, while the residual volume/total lung capacity ratio tended to increase after HSCT. Of 12 patients, 8 patients died and the median month from diagnosis to death was 33.5 months. Seven patients died of pulmonary or systemic infection, and one patient died due to relapse of the primary disease.ConclusionSevere restrictive lung defect could develop in selected cases in the late-phase after HSCT and could be a unique clinical entity with specific radiographical findings.
Highlights
Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3 months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation
idiopathic pneumonia syndrome (IPS) is a severe pulmonary complication occurring after HSCT, which was originally characterized by symptoms and signs of pneumonia, restrictive pulmonary function test abnormality, and alveolar injury without documented lower respiratory tract infection [8]
In case No 12, the episode of bacterial pneumonia reduced VC level, and pneumomediastinum and pneumothorax further decreased VC (Fig. 4e). These results indicate that the onset of pneumothorax and pulmonary infection contributed to the progressive decrease in restrictive pulmonary function and exacerbation of late onset severe restrictive lung defect, contributing to its poor prognosis. In this retrospective observational study of allogeneic HSCT recipients, we investigated the clinical features of patients with late-onset severe restrictive lung defect (%VC less than 60%) and identified 12 cases with characteristic radiological findings and pulmonary function changes
Summary
Late-onset noninfectious pulmonary complications (LONIPCs), which occur more than 3 months after allogeneic hematopoietic stem cell transplantation (HSCT), are major causes of morbidity and mortality after transplantation. IPS is a severe pulmonary complication occurring after HSCT, which was originally characterized by symptoms and signs of pneumonia, restrictive pulmonary function test abnormality, and alveolar injury without documented lower respiratory tract infection [8]. The time of onset for IPS ranges from 4 to 106 days and diffuse alveolar hemorrhage (DAH) occurs in early post-HSCT, with an indicated median onset time of 12–15 days [9]. It seems that LONIPCs generally do not include IPS, according to the ATS statement
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